Atypical anti-glomerular basement membrane disease

Megan L. Troxell, Donald C. Houghton

Research output: Contribution to journalArticle

13 Scopus citations

Abstract

Background Anti-glomerular basement membrane (anti-GBM) disease classically presents with aggressive necrotizing and crescentic glomerulonephritis, often with pulmonary hemorrhage. The pathologic hallmark is linear staining of GBMs for deposited immunoglobulin G (IgG), usually accompanied by serum autoantibodies to the collagen IV alpha-3 constituents of GBMs. Methods Renal pathology files were searched for cases with linear anti-GBM to identify cases with atypical or indolent course. Histopathology, laboratory studies, treatment and outcome of those cases was reviewed in detail. Results Five anti-GBM cases with atypical clinicopathologic features were identified (accounting for ∼8% of anti-GBM cases in our laboratory). Kidney biopsies showed minimal glomerular changes by light microscopy; one patient had monoclonal IgG deposits in an allograft (likely recurrent). Three patients did not have detectable serum anti-GBM by conventional assays. Three patients had indolent clinical courses after immunosuppressive treatment. One patient, untreated after presenting with brief mild hematuria, re-presented after a short interval with necrotizing and crescentic glomerulonephritis. Conclusions Thorough clinicopathologic characterization and close follow-up of patients with findings of atypical anti-GBM on renal biopsy are needed. Review of the literature reveals only rare well-documented atypical anti-GBM cases to date, only one of which progressed to end-stage kidney disease.

Original languageEnglish (US)
Pages (from-to)211-221
Number of pages11
JournalClinical Kidney Journal
Volume9
Issue number2
DOIs
StatePublished - Apr 1 2016

Keywords

  • Goodpasture's
  • anti-glomerular basement membrane disease
  • crescentic glomerulonephritis

ASJC Scopus subject areas

  • Nephrology
  • Transplantation

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